Dilemmas in the management of patients with vulval epithelioid sarcoma: a literature review

Abstract

Vulval sarcomas are an extremely rare type of soft tissue tumor, the principal properties of which are unpredictable symptoms and frequent recurrences. This review discusses the management options specific for cases of vulval epithelioid sarcoma. The PubMed, Scopus and Cochrane databases were systematically searched and 28 studies met the inclusion criteria for our narrative review. The mean age of the 31 included patients was 31 years (range: 17-84). Local excision (19 out of 31, 61.3%), radical vulvectomy (8 out of 31, 25.8%) and hemivulvectomy (4 out of 31, 12.9%) were the principal surgical treatments. Radiotherapy and chemotherapy were performed in 8 and 5 patients, respectively. Recurrence of the disease was present in 13 out of 31 (42%). The interval to recurrence ranged from 1 to 48 months. The main location of recurrences was the local tissues, the lymph nodes and the lung. The mean period of follow-up was 38.5 months (range: 2-146 months). Cure was considered to have taken place in 19 out of 31 (61.3%) patients; 10 out of 31 (32.6%) died, and 2 out of 31 (6.4%) at the end of follow-up were alive but not considered cured. The first and principal step for the proper treatment of vulval epithelioid sarcomas is awareness of their existence by the specialist involved. Extensive imaging is proposed for staging, while the creation of a national or international register of patients with this malignancy would enable a more consistent approach to its management.

Keywords: Management; Treatment strategy; Vulval epithelioid sarcoma; Vulval malignancy.